Subacute Sclerosing Panencephalitis (SSPE) in Toddlers and Young Children: A Case Series

SSPE is a neurodegenerative disorder caused by persistent defective or mutant measles virus. The disease has a gradual progressive course leading to death within 1-3 yrs or even early. SSPE is a disease of childhood and early adolescence. The classic age at presentation is 8-11 years and usually occurs after a latent period of average 6 years. Here we report 3 cases of SSPE in toddlers. Diagnosis was made on the basis of clinical presentation, EEG pattern and elevated CSF anti measles antibody titer as described in Dykan criteria for diagnosis of SSPE. Clinical presentation was very early with a relatively shorter latency and fatal progression. Two patients had past history of measles and all patients were immunized against measles. One patient died within 4 months of disease expression and 2 patients went into vegetative state within 3 months of disease onset. Bangladesh J Child Health 2020; VOL 44 (2) :114-117

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Atypical Presentation of Fulminating Subacute Sclerosing Panencephalitis: A Case Series

Neuropediatrics ◽

10.1055/s-0040-1715623 ◽

2020 ◽

Author(s):

Pranab K. Dey ◽

Arindam Ghosh

Keyword(s):

Measles Virus ◽

Subacute Sclerosing Panencephalitis ◽

Case Series ◽

Acute Disseminated Encephalomyelitis ◽

Initial Symptom ◽

Gait Abnormality ◽

First Case ◽

History Of ◽

Atypical Presentations ◽

Fulminant Type

AbstractSubacute sclerosing panencephalitis (SSPE) is a rare and progressive inflammatory disease of central nervous system due to aberrant measles virus with an outcome that is nearly always fatal. In acute fulminant SSPE, the disease rapidly evolves leading to death within 3 months of the diagnosis. We report here four cases of fulminant SSPE with atypical presentations, two of them presented at very early age with history of congenital measles infection in first case and gait abnormality as initial symptom in second case; acute disseminated encephalomyelitis (ADEM) with refractory seizures in third case, unilateral myoclonus with hemiparesis in fourth case at the onset of disease, respectively. The typical periodic electroencephalographic (EEG) complexes, elevated cerebrospinal fluid (CSF), and serum antimeasles antibodies in our patients led to the diagnosis of SSPE. A high index of clinical suspicion in fulminant type with awareness of atypical features, EEG, and CSF studies are of paramount importance in establishing its diagnosis.

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Low Psychosine in Krabbe Disease with Onset in Late Infancy: A Case Report

International Journal of Neonatal Screening ◽

10.3390/ijns7020028 ◽

2021 ◽

Vol 7 (2) ◽

pp. 28

Author(s):

Camille S. Corre ◽

Dietrich Matern ◽

Joan E. Pellegrino ◽

Carlos A. Saavedra-Matiz ◽

Joseph J. Orsini ◽

...

Keyword(s):

New York ◽

Enzyme Activity ◽

Clinical Presentation ◽

Neurodegenerative Disorder ◽

New York State ◽

Disease Onset ◽

Early Infancy ◽

Krabbe Disease ◽

Activity Levels ◽

Hematopoietic Stem

Krabbe disease (KD) is a rare inherited neurodegenerative disorder caused by a deficiency in galactocerebrosidase enzyme activity, which can present in early infancy, requiring an urgent referral for hematopoietic stem cell transplantation, or later in life. Newborn screening (NBS) for KD requires identification and risk-stratification of patients based on laboratory values to predict disease onset in early infancy or later in life. The biomarker psychosine plays a key role in NBS algorithms to ascertain probability of early-onset disease. This report describes a patient who was screened positive for KD in New York State, had a likely pathogenic genotype, and showed markedly reduced enzyme activity but surprisingly low psychosine levels. The patient ultimately developed KD in late infancy, an outcome not clearly predicted by existing NBS algorithms. It remains critical that psychosine levels be evaluated alongside genotype, enzyme activity levels, and the patient’s evolving clinical presentation, ideally in consultation with experts in KD, in order to guide diagnosis and plans for monitoring.

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Subacute Sclerosing Panencephalitis

Journal of Bangladesh College of Physicians and Surgeons ◽

10.3329/jbcps.v37i4.43352 ◽

2019 ◽

Vol 37 (4) ◽

pp. 205-208

Author(s):

Gobinda Chandra Banik ◽

Sakib Aman ◽

Farhana Sultana ◽

Syed Mohammad Arif

Keyword(s):

Cerebrospinal Fluid ◽

Virus Infection ◽

Antibody Titer ◽

Measles Virus ◽

Subacute Sclerosing Panencephalitis ◽

Abnormal Behavior ◽

Short History ◽

Altered Consciousness ◽

History Of ◽

Metachromatic Leucodystrophy

Subacute sclerosing panencephalitis (SSPE) is chronic progressive encephalitis of childhood and young adoloscent due to persistent measles virus infection. This case illustrates a 14 year old girl presented with short history of intellectual decline, abnormal behavior, myoclonus and altered consciousness with suggestive neuroimaging mimicking metachromatic leucodystrophy. Subsequently she was diagnosed to be a case of Subacute sclerosing panencephalitis (SSPE) on the basis of Electroencephalography (EEG) and Cerebrospinal fluid(CSF) measles antibody titer. J Bangladesh Coll Phys Surg 2019; 37(4): 205-208

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Clinical trends of scalp alopecia areata: a tertiary care hospital based observational study

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20200607 ◽

2020 ◽

Vol 6 (2) ◽

pp. 193

Author(s):

Pallavi Singh ◽

Milind A. Patvekar ◽

Bhavika Shah ◽

Alisha Mittal ◽

Asmita Kapoor

Keyword(s):

Family History ◽

Observational Study ◽

Alopecia Areata ◽

Tertiary Care Hospital ◽

Past History ◽

Tertiary Care ◽

Disease Onset ◽

Epidemiological Data ◽

Care Hospital ◽

History Of

Background: Alopecia areata is one of the commonest types of non-scarring alopecia involving the scalp and/or body. As there is paucity of recent epidemiological data from our country, this study was conducted to determine the latest clinical and epidemiological trends of scalp alopecia areata.Methods: A hospital-based observational study consisting of 100 cases of clinically diagnosed scalp alopecia areata who reported to the Dermatology OPD, Dr. D.Y. Patil Medical College, Pune, was conducted for a period of six months. Socio-demographic and clinical information was collected and clinical examination was performed on all patients. The data was evaluated using appropriate statistical methods.Results: Out of the 100 cases enrolled, males (64%) outnumbered females (36%). The commonest presenting age group was 21-30 years (44%). Disease onset was sudden in 80% patients and 59% cases had a progressive disease course. Majority (75%) had a disease duration of less than 3 months. Majority cases were asymptomatic (80%) with no precipitating factors (90%). Past history and family history of alopecia areata were present in 13% and 9% cases, respectively. Personal and family history of associated diseases were present in 27% and 22% patients, respectively. Most patients had single (61%), patchy (83%) lesions with occiput (45%) being the commonest initial site. Nail changes were present in 22% cases, of which pitting (13%) was the commonest nail finding.Conclusions: This study reflects the clinical profile of scalp alopecia areata in a tertiary care hospital.

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Childhood Pilomatrixoma: Case Series from a Single Center

Trends in Pediatrics ◽

10.5222/tp.2020.43534 ◽

2020 ◽

Author(s):

Begümhan Demir Gündoğan ◽

Fatih Sağcan ◽

Mehmet Alakaya ◽

Ferah Tuncel Daloğlu ◽

Elvan Çağlar Çıtak

Keyword(s):

Differential Diagnosis ◽

Medical Records ◽

Clinical Presentation ◽

Case Series ◽

Neck Region ◽

Head And Neck Region ◽

Common Causes ◽

Institutional Experience ◽

History Of ◽

Associated Conditions

INTRODUCTION: The aim of this study is to describe our institutional experience with pilomatrixoma in children, specifically examining its clinical presentation, associated conditions, radiological and pathological findings and attract attention to differential diagnosis for this tumor. METHODS: The medical records of 52 patients were reviewed retrospectively. RESULTS: There were a total of 62 tumors in 52 children. The median age at excision was 9.5 years old. Tumors were predominantly located in head and neck region (48.4%). One patient had a family history of pilomatrixoma. One patient had Turner Syndrome and one had Tuberous Sclerosis complex. Fifty-four lesions were examined by ultrasonography (USG). Pilomatrixoma was considered in the differential diagnosis in eight patients (15.3%) by a radiologist. DISCUSSION AND CONCLUSION: Pilomatrixoma is one of the most common causes of superficial masses in children. It should be kept in mind for differential diagnosis in children with superficial masses.

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Vaping in today’s pandemic: E-cigarette, or vaping, product use–associated lung injury mimicking COVID-19 in teenagers presenting with respiratory distress

SAGE Open Medical Case Reports ◽

10.1177/2050313x20969590 ◽

2020 ◽

Vol 8 ◽

pp. 2050313X2096959

Author(s):

Daphne O Darmawan ◽

Kriti Gwal ◽

Brian D Goudy ◽

Sanjay Jhawar ◽

Kiran Nandalike

Keyword(s):

Severe Acute Respiratory Syndrome ◽

Lung Injury ◽

Respiratory Distress ◽

Pediatric Patients ◽

Clinical Presentation ◽

Case Series ◽

Product Use ◽

Abdominal Symptoms ◽

History Of ◽

High Index Of Suspicion

The clinical presentation of children and adolescents infected with severe acute respiratory syndrome coronavirus 2 can range from asymptomatic to mild or moderate manifestations. We present a case series of three adolescents who presented during the coronavirus disease 2019 (COVID-19) pandemic with symptoms concerning for COVID-19, including fever, abdominal symptoms, cough, respiratory distress, and hypoxemia. Their laboratory results showed elevated inflammatory markers that are also commonly seen in COVID-19. The chest imaging studies mimicked COVID-19 with non-specific ground glass opacities and interstitial prominence patterns. However, severe acute respiratory syndrome coronavirus 2 testing was negative and further questioning of these adolescents and their parents revealed a history of vaping marijuana-related products leading to the eventual diagnosis of e-cigarette, or vaping, product use–associated lung injury. Our patients were successfully treated with corticosteroids. The providers caring for pediatric patients, especially adolescents, should continue to have a high index of suspicion for e-cigarette, or vaping, product use–associated lung injury in patients presenting with unexplained respiratory failure, while ruling out COVID-19.

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Role of Carbamazepine in the Symptomatic Treatment of Subacute Sclerosing Panencephalitis: A Case Report and Review of the Literature

Case Reports in Neurological Medicine ◽

10.1155/2013/327647 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Sandhya Ravikumar ◽

John Ross Crawford

Keyword(s):

Clinical Presentation ◽

Vegetative State ◽

Subacute Sclerosing Panencephalitis ◽

Symptomatic Treatment ◽

The United States ◽

Review Of The Literature ◽

First Line ◽

Epileptiform Discharges ◽

The Subject

We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

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ADHD, ODD, and CD: Do They Belong to a Common Psychopathological Spectrum? A Case Series

Case Reports in Psychiatry ◽

10.1155/2012/520689 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 3

Author(s):

Sayanti Ghosh ◽

Mausumi Sinha

Keyword(s):

Conduct Disorder ◽

Past History ◽

Case Series ◽

Externalizing Disorders ◽

Developmental Pathways ◽

Hyperactivity Disorder ◽

Oppositional Defiant ◽

Symptom Profile ◽

History Of ◽

Shared Risk

Purpose of Research. Numerous studies have reported comorbidities, overlapping symptoms, and shared risk factors among cases of attention deficit hyperactivity disorder (ADHD), oppositional defiant disorder (ODD) and conduct disorder (CD). We present three adolescent males aged 13–16 years with conduct disorder having past history of ADHD and ODD.Principal Result. The symptom profile especially in domains of aggression, hostility, and emotionality as well as the manner of progression from ADHD to ODD and CD in the above cases shows a similar pattern.Conclusion. These common developmental pathways and overlapping symptoms suggest the possibility of a common psychopathological spectrum encompassing the three externalizing disorders.

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A Case Report of Non-Reactive Psychosis in a COVID-19 Patient

RECENT ADVANCES IN PATHOLOGY AND LABORATORY MEDICINE ◽

10.24321/2454.8642.202104 ◽

2021 ◽

Vol 7 (1&2) ◽

pp. 11-13

Author(s):

Manjulika Debnath ◽

Keyword(s):

Family History ◽

Clinical Presentation ◽

Psychiatric Symptoms ◽

Past History ◽

Psychotic Symptoms ◽

Antigen Test ◽

First Onset ◽

History Of ◽

Coronavirus Infection ◽

Anxiety Depression

Background: COVID-19 causes reactive psychiatric symptoms like anxiety, depression, insomnia etc besides significant inflammatory response. A number of COVID-19 patients are found to present with complex neuropsychiatric syndromes, including the first onset of psychosis, that seem to be directly related to brain damage in the context of COVID-19. Most cases of psychotic disorder in COVID-19 patients are being found in individuals with no personal or family history of mental illness. Case Description: A 33-year-old man presented with acute restlessness, agitation, wandering, vandalizing things, suspiciousness, hallucinatory behaviour, decreased sleep for 3 days duration. There was no past history or family history of any psychiatric illness. There was a history of mild grade fever 7 days before the onset of the psychotic symptoms. The patient was uncooperative, irritable, and had hallucinatory behaviour. Rapid Antigen Test (RAT) was positive. The patient responded to injectable antipsychotic haloperidol which was later changed to Tab Olanzepine 5 mg. The patient developed no COVID symptoms and no psychotic symptoms were seen further. Conclusion: There is a possibility of a psychosis break as a COVID-19 clinical presentation, suggesting potential participation of inflammatory and autoimmunologic phenomena triggered as a response to the coronavirus infection.

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Diagnosis of Huntington Disease

Clinical Chemistry ◽

10.1373/49.10.1726 ◽

2003 ◽

Vol 49 (10) ◽

pp. 1726-1732 ◽

Cited By ~ 68

Author(s):

Russell L Margolis ◽

Christopher A Ross

Keyword(s):

Huntington Disease ◽

Clinical Presentation ◽

Neurodegenerative Disorder ◽

Diagnostic Testing ◽

Disease Onset ◽

Test Results ◽

Genetic Test Results ◽

Essential Components ◽

Trinucleotide Expansion ◽

Expansion Mutation

Abstract Background: Huntington disease (HD) is a rare, progressive, and fatal autosomal dominant neurodegenerative disorder, typically of adult onset. Methods: We reviewed the literature concerning the molecular diagnosis of HD. Results: The discovery of the genetic etiology of HD, a trinucleotide expansion mutation on chromosome 4p, has led to the development of increasingly reliable and valid diagnostic tests that can be applied to symptomatic patients, individuals at risk for HD but currently asymptomatic, fetuses, and embryos. However, the unstable nature of the HD mutation, the lack of effective treatments for HD, the mid-adulthood age of disease onset, and the existence of disorders with the same clinical presentation but different etiology all complicates diagnostic testing. Conclusion: Conscientious laboratory work, knowledgeable interpretation of genetic test results, and the availability of pre- and posttest counseling are essential components of HD diagnosis.

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